Background: Lymphomas of the ocular adnexa are heterogeneous and demonstrate a wide range of clinical, histological, immunohistochemical and molecular genetic characteristics.
Aim: The aim of this article is to give an overview of the interdisciplinary diagnostics and individually adapted lymphoma subtype-based therapy.
Diagnostics: Depending on the lymphoma localisation, i.e. whether in the eyelid, the conjunctiva or in the orbit, a photograph or a radiological scan is required to record the tumor extent. Visual function is more likely to be impacted when the lymphoma arises in the posterior orbit, close to the optic nerve and imaging diagnostics are therefore necessary. Histological investigations are essential for confirming the lymphoma diagnosis and give information about the particular subtype, which in turn will determine subsequent patient management, Clinical staging investigations for determining the systemic extent of the lymphoma manifestation (e.g. imaging, blood analyses as well as bone marrow biopsy) are mandatory.
Therapy: External beam radiation, local and systemic chemotherapy or in some cases antibiotics are treatment options after surgical excision in isolated ocular adnexal lymphoma. The TNM classification of the American Joint Committee on Cancer or the Ann Arbor staging system, as well as the guidelines of the German Society of Hematology and Medical Oncology are all tools to aid the choice of the appropriate individually adapted therapy for systemic disease, which includes psycho-oncological care.